Locked-in syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at October 2, 2016
StartDiseasesLocked-in syndrome

Locked-in syndrome refers to a patient who has no ability to speak or move but may be able to control eye movement. With this movement, the patient may be able to express themselves using tools designed to read eye movements. These tools can then transcribe the letters or words selected by the eye movement and give voice to the locked-in patient. Locked-in syndrome is also referred to as pseudocoma.

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Definition & Facts

It is different from a true coma because the patient develops an awareness of their surroundings and does not lose cognitive function. The patient has no ability to speak, move or express themselves except for their eye movements. Once diagnosed, life expectancy can be extended for patients who develop a system of communication and the chance to connect.

While movement or regular speech may never again by possible, the ability to communicate can improve the quality of life for a locked-in patient. With the right computer-assisted tool, the patient may be able to speak through a computer, or even control a mechanized chair.

Symptoms & Complaints 

Locked-in syndrome causes the inability of the patient to speak or move. Due to paralysis and impairment of major motor functions, those who suffer locked-in syndrome cannot speak, swallow (dysphagia), or control any of their muscle groups.

Often, those suffering from locked-in syndrome are completely unconscious for a time after the traumatic brain injury, but eventually are able to open their eyes. Brain function for most of these patients returns to previous levels, but they have no ability to communicate but for eye movements.

Causes

This condition is generally caused by severe damage to a section of the brainstem called the pons. The damage to the brainstem breaks or severely damages the connection between the brain and the spinal cord, leaving the patient paralyzed.

Most patients suffering locked-in syndrome develop the condition after suffering a stroke, which causes a lack of blood flow to the brain. In other cases, the damage can be caused by a brain hemorrhage or brain aneurysm.

In rarer cases, the brainstem damage can be caused by a blow or traumatic injury. Additionally, conditions such as amyotrophic lateral sclerosis (ALS) can lead to locked-in syndrome.

In rarer cases, inflammation of the spinal sheath can lead to paralysis. Illnesses including meningitis can cause this damage, as can adverse drug reaction and drug overdose.

Diagnosis & Tests

Clinical diagnosis of locked-in syndrome involves working to communicate with the patient and encouraging them to use eye movements to respond to questions. Brain activity may be monitored with an EEG or electroencephalogram.

Nerve conduction studies may be undertaken to attempt to determine the source of the paralysis and any further damage to the brain. A magnetic resonance imaging (MRI) scan can also be done to review the function and structure of the brain and body as well as assess their blood vessels to determine the source of the brainstem damage. This medical imaging test, along with computed tomography (CT) scans can also assess the presence of causal tumor.

This condition may not be recognized for months and even years, during which a perfectly conscious individual is essentially imprisoned in their own body, unable to communicate their consciousness.

Treatment & Therapy

While communication and self-expression are critical when caring for a patient with locked-in syndrome, a great deal of specialized care must be taken to protect the patient from life-threatening symptoms caused by the paralysis suffered because of the brain injury. Treatment for complete paralysis will most likely include:

Once the physical needs of the patient have been met, therapeutic communication treatments for the locked-in patient include the use of eye tracking devices using an infrared reader. This eye reader may be attached to a computer that can speak for the patient. These tools can also help the patient control a motorized wheelchair.

Should the patient develop abilities beyond eye movement, any motor control that can be regained regarding speaking and swallowing should be pursued diligently. Regaining the ability to speak and swallow can greatly improve the quality of life for a locked-in patient.

Prevention & Prophylaxis

Because locked-in syndrome can be caused by stroke or brain hemorrhage, careful monitoring of all stroke risks should be undertaken by anyone with a history of high blood pressure (hypertension) or habits such as smoking.

Dietary improvements such as increasing plant-based foods, cutting back on salt and reducing sugar intake can reduce the risk of stroke. Additionally, starting an exercise program at any age can keep the brain healthier.

Diabetes mellitus must be managed, smoking must be ceased, and excessive alcohol intake addressed, as these disorders and behaviors can increase the risk of stroke.

Because the condition can develop after drug overdose, it's important to note that addiction treatment is critically important for any and all users of illegal or improperly used prescription drugs. Long-term exposure to improperly used drugs, no matter the source, adds to the risk of unintentional overdose and may lead to this life-changing condition. Drug users must be encouraged to participate in treatment programs designed to aid in breaking the cycle of addiction.

Additional methods of prevention can include the wearing of helmets while boxing, bicycling or motorcycle riding in order to prevent traumatic brain injury. If a concussion is suspected, it is important to seek treatment immediately. While damage to the brain stem is rare in sporting accidents, protecting the brain from blows can reduce the risk.

Locked-in syndrome is a very rare condition. However, with careful observation and detailed therapy designed to help the patient regain the ability to communicate, the isolation of paralysis in combination with the inability to speak can be overcome among certain patients.